Cystic Fibrosis – Symptoms, Diagnosis, and ICD-10 Codes

Cystic Fibrosis – Symptoms, Diagnosis, and ICD-10 Codes Physicians need to use the right ICD-10 codes to report cystic fibrosis as well as use additional codes to identify any infectious organism present.

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Cystic fibrosis is a devastating genetic disease that affects thousands of people across the globe.

It causes persistent lung infections and affects the ability to breathe over time. Pulmonary disease is the most common cause of death in patients with cystic fibrosis. According to the Cystic Fibrosis Foundation, the U.S. is one of the countries with the highest incidence of cystic fibrosis, with about 30,000 people currently living with the disease and between 900 and 1,000 new cases diagnosed every year. Medical coding and medical billing services are available to help physicians report diagnosis and treatment of this inheritable progressive disease.

Symptoms and Causes Cystic fibrosis is inherited and the fetus receives the defective gene from both parents. The gene affects the cells that produce mucus, sweat and digestive juices. Theses secretions become sticky and thick and block the tubes, ducts and passageways, especially in the lungs and pancreas. The condition causes severe damage to the lungs, digestive system and other organs.

All newborns are screened for cystic fibrosis in the US. The signs and symptoms of this complex disease vary, depending on the severity of the disease. In some cases, symptoms do not appear until adolescence or adulthood. The symptoms of the condition are as follows: -

Salty sweat

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Persistent cough with sputum

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Wheezing and breathing problems

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Recurrent lung infections

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Stuffy nose

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Inability to exercise

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Foul-smelling, greasy stools

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Poor growth or weight gain

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Intestinal blockage, especially in newborns

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Severe constipation

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Bloating

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Nausea

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Loss of appetite

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Diagnosis and Treatment Diagnosing cystic fibrosis is a multistep process. Various tests are used to diagnose and monitor the disorder: -

A sweat chloride test is standard to check for higher-than-normal amount of salt

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The standard screening blood test for newborns checks for higher than normal levels of a chemical (Immuno reactive trypsinogen or IRT)

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Other tests performed to monitor the condition after diagnosis include imaging (such as chest X-ray, CT, MRI), pulmonary function testing (spirometry), sputum culture, and organ function test

Early diagnosis will allow treatment to be started earlier and ease symptoms and reduce complications.

Efforts are on to find a cure for cystic fibrosis. However, challenging symptoms of the condition can be effectively managed through regular physiotherapy or PT (particularly chest PT), exercise, mucus-thinning medication, antibiotics, airway-clearing methods and devices. These interventions can help patients to lead longer and full lives.

ICD-10 Codes for Cystic Fibrosis

The ICD-10 codes for cystic fibrosis fall under metabolic diseases (E70-E88) in Chapter 4 Endocrine, nutritional and metabolic diseases. Physicians can choose from the following codes to indicate a diagnosis of cystic fibrosis for reimbursement purposes: 

E84.0 Cystic fibrosis with pulmonary manifestations

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E84.11 Meconium ileus in cystic fibrosis

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E84.19 Cystic fibrosis with other intestinal manifestations

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E84.8 Cystic fibrosis with other manifestations

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E84.9 Cystic fibrosis, unspecified

Coding guidelines state that an additional code should be reported to identify any infectious organism present.

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A 2016 ICD-10 Monitor article cites the Coding Clinic, Fourth Quarter 2002 on sequencing guidance with regards to cystic fibrosis and pulmonary manifestation: o The circumstances of the admission will determine the principal diagnosis: If the specific manifestation (such as Pseudomonas pneumonia (J15.1)) is the reason that necessitated the patient’s admission to the hospital, it would be sequenced as the principal diagnosis.

Cystic fibrosis may be listed as the principal diagnosis if the physician determines it is the reason for the admission. o The Medicare Severity Diagnosis Related Group (MS-DRG) can vary based on the circumstances of admission determine the principal diagnosis: If pseudomonas pneumonia with cystic fibrosis is the reason for admission, the MS-DRG is 177; if the admission is due to unspecified cystic fibrosis, the MS-DRG is 164. o The cystic fibrosis will affect the MS-DRG assignment if it qualifies as a complication/comorbid condition.

Correctly capturing a patient’s clinical condition influences how hospitals are reimbursed under the Medicare severity-adjusted DRG system. Diagnosis should be coded to the highest level of specificity.

Complete documentation in the medical record is crucial for accurate coding. An experienced medical billing company will have skilled coding professionals who can assign diagnosis codes to the highest degree of specificity documented. Physicians can work with these coders ensure complete and accurate documentation, code assignment, and reporting of diagnoses as well as procedures.

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